Malignant paraganglioma of the retroperitoneum with lung metastases: a 13-year survivor after radical surgery.

We present a 23-year-old patient with extra-adrenal retroperitoneal paraganglioma with lung metastases who was successfully treated by complete removal of the tumour. Lung metastases were the first manifestation of the disease, and an abdominal computed tomography scan showed a large mass in the retroperitoneum with marked contrast enhancement. Angiography demonstrated a hypervascular mass with many feeding arteries, but vascular invasion was not apparent. The retroperitoneal tumour was resected completely followed by resection of lung metastases after 1 month of observation. The patient was disease free for 13 years after this radical surgery. The survival rate in patients with retroperitoneal paraganglioma with lung metastasis is low, and this case represents the longest surviving period reported in the literature. These tumours are usually large and located in the para-aortic region, and hence resection is sometimes challenging. We believe that a complete and meticulous surgical procedure is a prerequisite for long survival from this rare disease.

Surgery plus chemotherapy compared with surgery alone for localized squamous cell carcinoma of the thoracic esophagus: a Japan Clinical Oncology Group Study--JCOG9204.

PURPOSE: We performed a multicenter randomized controlled trial to determine whether postoperative adjuvant chemotherapy improves outcome in patients with esophageal squamous cell carcinoma undergoing radical surgery. PATIENTS AND METHODS: Patients undergoing transthoracic esophagectomy with lymphadenectomy between July 1992 and January 1997 at 17 institutions were randomly assigned to receive surgery alone or surgery plus chemotherapy including two courses of cisplatin (80 mg/m2 of body-surface area x 1 day) and fluorouracil (800 mg/m2 x 5 days) within 2 months after surgery. Adaptive stratification factors were institution and lymph node status (pN0 versus pN1). The primary end point was disease-free survival. RESULTS: Of the 242 patients, 122 were assigned to surgery alone, and 120 to surgery plus chemotherapy. In the surgery plus chemotherapy group, 91 patients (75%) received both full courses of chemotherapy; grade 3 or 4 hematologic or nonhematologic toxicities were limited. The 5-year disease-free survival rate was 45% with surgery alone, and 55% with surgery plus chemotherapy (one-sided log-rank, P =.037). The 5-year overall survival rate was 52% and 61%, respectively (P =.13). Risk reduction by postoperative chemotherapy was remarkable in the subgroup with lymph node metastasis. CONCLUSION: Postoperative adjuvant chemotherapy with cisplatin and fluorouracil is better able to prevent relapse in patients with esophageal cancer than surgery alone.

A case of primary gastric small cell carcinoma with a rare pattern of lymph node metastasis.

We report a very rare case of primary gastric small cell carcinoma (GSCC) that was accompanied with gastric tubular adenocarcinoma. A male in his 60s had an elevated tumor with a central ulceration in the middle stomach. The patient underwent a distal gastrectomy with lymph node dissection. The pathological examination showed two separated lesions of the stomach, which contained the components of primary GSCC and primary gastric tubular adenocarcinoma. Immunohistochemical (IHC) examination demonstrated that the tumor cells in the small cell carcinoma stained positive for synaptophysin, chromogranin A, and neural cell adhesion molecule (NCAM). GSCC cells and adenocarcinoma cells independently metastasized to each regional lymph node. Further studies on the biological behavior of individual tumors may allow the development of new treatment strategies for GSCC.

Ruptured pancreaticoduodenal artery aneurysm with acute gangrenous cholecystitis: a case report and review of the literature.

We have experienced a very rare case of ruptured pancreaticoduodenal artery aneurysm with acute gangrenous cholecystitis. A 67-year-old male complaining of epigastralgia was admitted to our hospital. Ultrasound sonography demonstrated acute cholecystitis and cholecystolithiasis. Computed tomography scan showed the findings of acute cholecystitis and retroperitoneal mass. Emergency laparotomy revealed an acute gangrenous cholecystitis and a retroperitoneal hematoma around the second portion of the duodenum. Cholecystectomy was performed, however, the bleeding vessel was not identified. The patient bled again from the abdomen on the 6th postoperative day. A postoperative angiography indicated an inferior pancreaticoduodenal artery aneurysm. A resection of the aneurysm was performed following the angiography. Pancreaticoduodenal artery aneurysms are uncommon and ruptured pancreaticoduodenal artery aneurysms result in fatal hemorrhage and high mortality. We reviewed the previously reported cases and discussed the suitable and expeditious diagnosis and management of the pancreaticoduodenal artery aneurysms.

Primary small cell carcinoma of the breast.

Primary small cell carcinoma of the breast is a very rare disease, and only a few case reports have described small cell carcinoma of the breast that responds to chemotherapy. Here, we report a case of primary small cell carcinoma of the breast that was treated with surgery and chemotherapy for postoperative local recurrence in the chest wall and metastasis to the liver. The metastatic lesions showed a partial response (PR) to carboplatin and irinotecan, but did not respond to subsequent Taxotere and doxifluridine (5'-DFUR) treatment. We then treated the metastatic lesions with CBDCA and etoposide (VP-16), and were able to stop disease progression. Small cell carcinoma of the breast is as aggressive as its pulmonary counterpart. Therefore, the best therapy for primary small cell carcinoma of the breast may be surgery followed by adjuvant therapy similar to that recommended for small cell lung carcinoma.

Clinical significance of perioperative immunonutrition for patients with esophageal cancer.

BACKGROUND: We hypothesized that preoperative and/or postoperative enteral immune-enhanced formulas that are supplemented with arginine, omega-3 fatty acids, and RNA may reduce postoperative complications in patients undergoing esophagectomy for thoracic esophageal squamous cell carcinoma (ESCC). METHODS: Forty patients who underwent esophagectomy were divided into three groups: group A (n = 20) received a control enteral diet (Erental) through the jejunostomy after surgery. group B (n = 6) received an enteral diet supplemented with immune-enhancing substrates (Impact) containing arginine, omega-3 fatty acids, and RNA after surgery. group C (n = 14) received the impact before and after surgery. RESULTS: Lymphocyte counts in group C on postoperative day (POD) 7 were somewhat higher than that in group A (p = 0.07) and significantly higher than in group B (p = 0.03). Furthermore the incidence of incisional wound infection in group C was significantly lower than that in group A (p = 0.03). Moreover, the duration of postoperative systemic inflammatory response syndrome (SIRS) was significantly shorter in group C than in group A (p < 0.05). CONCLUSIONS: This study reveals that the perioperative immune-enhanced formula may be superior to postoperative control enteral formulas in terms of reducing surgical wound infection and postoperative SIRS, which may result in serious postoperative complications for patients who have undergone esophagectomy.

Pretreatment plasma fibrinogen level correlates with tumor progression and metastasis in patients with squamous cell carcinoma of the esophagus.

BACKGROUND: Hypercoagulation has been reported to be associated with tumor progression and a poor prognosis in various carcinomas. In this study, we examined fibrinogen levels in pretreated patients with esophageal squamous cell carcinoma (ESCC) and assessed its correlation with clinicopathological factors and prognosis in patients with ESCC. METHODS: Pretreatment fibrinogen levels were examined prior to surgery or other treatments (e.g. endoscopic mucosal resection and chemoradiotherapy [CRT]) in 105 patients with primary ESCC. We investigated the association of fibrinogen levels with clinicopathological background factors and the survival of ESCC patients. RESULTS: The plasma fibrinogen concentration (PFC) ranged from 209.4 to 781.6 mg/dL. Pretreatment PFC correlated significantly with the depth of invasion (T factor). There also existed a significant correlation between higher fibrinogen levels and lymph node metastasis (N factor) and distant organ metastasis. Patients with a higher fibrinogen level experienced a significantly worse overall survival (P = 0.006). Fibrinogen levels strongly correlated with platelet counts, white blood cell counts and tumor length. Pretreatment PFC were observed to have a significant correlation with CRT responsiveness in ESCC patients in stages II and III (P = 0.005). CONCLUSION: This study revealed that higher levels of fibrinogen correlated with tumor progression, metastasis and poor responsiveness to CRT in ESCC patients.